Charcot-Marie-Tooth Guide for Orthotics

At Ortho Rehab Designs we carefully evaluate your specific mechanical profile. We are American Board Certified in Orthotics and Prosthetics. We stress that if you are to be treated by an orthotist for CMT that they should be board certified in the discipline of Orthotics. We feel this is essential in order for basic standards to be met. We will work with your physician or physical therapist in order to ascertain muscle strengths and physical profiles that will help in the design of your orthosis. We pride ourselves in listening to the needs of each patient. Each device is designed for that specific patient's needs and physical mechanical profile. Our approach is straightforward and honest. We will always be attentive to your needs and goals.

I established Ortho
Rehab Designs in Las Vegas in 1991. My goal was a focus on hard
to fit patients that required creating stability and prevention
of further deformity. My primary goals have always been to increase
function and endurance for my patients. Being born with a foot
deformity and defects in the skeletal structure of my right foot,
forced me to be an orthotic wearer since the walking age of one.
Throughout my professional life I have always sought out more
ways to control my foot structurally and maintain as much function
as possible. I have found that the combination of carbon graphite
and silicone is the best way to achieve this with maximal correction
and maximal comfort. When you visit Ortho Rehab Designs I would
be glad to show you my foot, and can give you a living example
of what can be done. While I am not neurologically impaired, my
foot deformity is at a severe level. This has led me to develop
bracing systems that can prevent further joint deterioration and
maintain function. As my goals are similar to yours, I do not
want to have joints and muscles that deteriorate with age. The
goal is to maintain as much of what you have throughout your entire
life.

As you are currently
researching bracing for CMT, you will have many questions. We
will try and address the most common ones here. We urge you to
e-mail us with any questions or concerns you may have.

Question:
I have a high steppage gait. Will this disappear with braces?Answer:
Yes, but only if you are braced in proper alignment.

Question:
I feel off-balance in my current AFO's. Why is this?Answer:
It is generally due to a poor fit, or poor fit in conjunction
with poor
alignment.

Question:
I feel extremely tired after walking a long distance, is this
related to
my braces? Answer:
This is a strong indicator that you are not getting the benefits
from
an energy return system. Or you might be walking with gait
compensations that are causing early fatigue.

Question:
With my current brace I am getting severe pain in my foot from
the
footplate. Why is this?Answer:
Generally, this is due to poor mold rectifications. Some
practitioners overcorrect, or there simply might not be any
correction at all. This is something that needs to be checked.

Question:
What are floor reaction forces?Answer:
The reaction from the floor as an object, such as a ball or a
foot, or
a braced limb, strikes it.

Question:
With my current braces I try to get to a faster walking speed,
and I
have trouble sometimes as there is a hesitation when my foot tries
to roll over. Why is this?Answer:
It can be because: 1) there is not enough energy loading, and
consequently not enough energy released by your brace. 2) if
your brace has a full length footplate, and you have strong
quadriceps muscles, you will not be able to bend your metatarsals
enough. In addition, a full length footplate with a person who
has good quadriceps, causes a tremendous amount of floor
reaction. Floor reaction used in bracing systems is generally
done
for the purpose of creating an extension force at the knee, and
preventing the knee from buckling. This is not a desirable force
system to use with a person who has good quadriceps.

Question:
How fast should I be walking?Answer:
The average human walking speed is 3.0 miles per hour.

At Ortho Rehab Designs
we provide CMT patients with videographic assessment on request.
However, if you will be seeing us from out-of-town, a videographic
assessment is necessary as a tool to pre-evaluate you before you
come to our office. From this assessment we can get a preliminary
evaluation and determine what gait deviations and deformities
your body is experiencing and this will allow us to make orthotic
recommendations based on that. Please keep in mind our evaluations
are limited to what we can assess on the videotape and computer.
You will find that in the orthotic field some facilities may use
similar videographic assessments and make assumptions that certain
ligaments are stressed or that certain bony deformities are present.
We stress that this cannot be ascertained medically unless a patient
has had an x-ray or MRI. Only x-rays can determine deviations
in the skeletal structure, and only MRI's can determine ligamentous
damages; with medical certainty.

Systematic analysis
is a valuable clinical tool for determining the nature and severity
of the patient's condition with skeletal or neuromuscular deviations
or deficiencies. This also helps assess the adequacy of orthoses
and other aids intended to assist in achieving a more normal ambulation.(1)

Many gait deviations
are created by the neuromuscular patterns produced from CMT. These
are a result of nerve loss and muscular weakness and ligamentous
laxity. Some of the more difficult gait deviations you might have
noticed on yourself or a member of your family might be as follows:

When we do a videographic
gait analysis of a patient we look for these gait deviations.
This helps us assess your walking pattern, and how the orthosis
will need to be configured to help normalize these deviations.
This evaluation gives us a preliminary guideline on what to look
for, and will ultimately be checked in the diagnostic stage.

At Ortho Rehab Designs
we specialize in energy loading, or what is also known as Dynamic
Release bracing. Our bracing designs for CMT are all mostly based
on dynamic release. We are the original inventors of the Double
Helix AFO. In addition, we have just recently introduced our newest
invention, the Helios Orthosis. Helios stands for
Helical Energy Loading Integrated Orthotic System.

The Helios incorporates
an extremely high modulous carbon graphite composite that creates
a very high level of energy loading and dynamic release. In addition,
the Helios incorporates a footplate using a custom designed
silicone padding system. Most other braces that you will see in
the orthotic marketplace use materials known as plastazote or
pelite. With plastazote or pelite you will get the intended initial
correction that you desire to maintain your foot in proper alignment.
However, within as little as a few weeks plastazote and pelite
start to compress from normal weight bearing. As it compresses
you will lose your initial structural alignment.

The reason we use silicone
is because silicone maintains its original durometer insuring
that you maintain the initial alignment you were corrected in,
and in addition, silicone is much kinder to your skin. You can
tolerate greater loads and feel more comfortable throughout the
day using silicone. I have used silicone on my own foot and this
is what led me to realize its value, and to incorporate it into
my bracing systems.

We
specialize in bracing for patients who have neuromuscular
diseases. Most of our CMT patients come to Las Vegas from
out-of-state. We have many patients using our bracing systems
who have had them a long time and are doing quite well. We
monitor their success as time goes by. We would be happy to
give you their names for referral so you can find out for
yourself the improvements our bracing systems have made for
them, and the professional care they received at our facility.
We will provide you with follow-up care as long as you need
it.

If you were just recently
diagnosed with CMT, you might be very concerned about what to
expect. As the disease progresses, symmetrical muscular atrophy
and weakness become apparent in the peroneal nerve muscles and
toe extensors.(2) Subsequently, the disease may advance to involve
the tibialis anterior muscles, in which case there is a bilateral
drop foot gait.(3)

This is a question
sometimes asked by CMT patients, although many CMT patients know
what type they have. The following is just a brief outline to
help you to become more familiar with what you have. If you know
the type you have when you call us or send in a videotape for
assessment, we will automatically know what to look for in your
correction system.

CMT Type 1A pes cavus or
high arch foot

Patients with CMT experience
muscle weakness, or an imbalance of muscle strength which causes
a pes cavus foot. In this type of foot the arch is raised into
a position that is classified as a clinical deformity. The muscle
that is responsible for this is the tibialis posterior. The forefoot
adducts in what is an inward angulation. The back of the foot,
or the hindfoot, usually goes into a varus position. This profile
needs very careful analysis for proper correction because many
times there is what we call a calcaneal varus contracture. Therefore,
it is necessary to address the calcaneal varus contracture as
a separate component. In these situations, we would like as much
input from your physical therapist or physician as possible.

The pes cavus foot
usually has four basic characteristics:
1. An unusually high longitudinal arch.
2. Toes that are clawed.
3. Prominent metatarsal heads.
4. Foot is shorter than normal.

The clawing of the toes is due to a contracture of the toe extensors.(4)

CMT1 is the most commonly
diagnosed type. Patients are inclined to manifest a pes cavus
deformity. In addition to the foot angulation if left uncorrected,
the alignment will affect the knee. Many patients find as soon
as they have heelstrike with the floor, they feel their foot and
knee rolling outward. As such, many patients that are affected
by the pes cavus foot have a very narrow walking base. Patients
have told me that other practitioners have instructed them to
walk with a wider gait. Although this sounds reasonable, it will
only encourage awkwardness and more compensations by the rest
of your muscles. When addressed properly, the pes cavus foot with
corrected alignment will reduce fatigue, thereby allowing greater
velocity and more endurance or increased walking time.

CMT
Type 2

The
pes planus foot is very familiar to me as I was born with
a pes planus third degree deformity. However, the CMT2 patient
with a pes planus foot, has ligamentous laxity, in contrast
to a pes planus foot that has a fixed or rigid deformity,
such as my own foot. Fortunately for CMT patients, a flexible
pes planus foot is easy to correct in comparison to a fixed
or rigid pes planus. The pes planus is also known as a valgus
deformity or where the foot goes into the

The pes planus foot,
or more commonly known as the flat foot.

position of eversion.
Most CMT patients will find it interesting to learn that a large
percentage of the general population (who do not have CMT), suffer
from pes planus feet. A major distinction between CMT patients
and other patients who have pes planus, is that with CMT there
is a foot drop or peroneal neuropathy, where the foot does not
dorsiflex due to a weakness of the anterior tibialis muscle.

The anterior tibialis
muscle is a large muscle on the front of your leg which dorsiflexes
or lifts your toes up. With a pes planus foot the calcaneus is
usually in a valgus position and the forefoot abducts (points
outward). Ideally we want to do the reverse, adduct the forefoot,
elevate the arch out of the pes planus position, and maintain
that new corrected alignment with stability. If this is done properly
it will reduce fatigue, pain, and allow you to increase your walking
time. I stress that silicone is critical when doing this kind
of correction.

As a sufferer from
a pes planus birth defect, I have a lifetime of experience dealing
with it. If I see you here in my office as a patient I would be
glad to show you my foot, the carbon graphite device that I use
to correct it, and can tell you what to expect. When I first incorporated
my silicone correction system, I was the first patient to test
it out. After using this for two to three months I realized the
benefits and then started incorporating it into my new Helios
Orthosis. I now have many patients using the silicone and they
can tell you of its benefits.

Pes planus, or pes
valgo planus, is a deformity producing a severe flat foot. This
deformity mostly takes place at one particular joint, which is
the talocalcaneal articulation. When there is a valgus deformity
at this joint, there are usually three separate components that
are put into effect:
1. The calcaneus has a valgus position.
2. The head of the talus angulates downward.
3. In relation to the hindfoot, the forefoot is totally abducted.

In addition, with this
deformity the achilles tendon is normally pulled laterally. This
is because of the outward rotation of the calcaneus. The outward
rotation of the calcaneus displaces the line of pull of the achilles
tendon. The primary muscles that plays a role in this is the gastrocnemius
and soleus. The gastrocnemius and soleus muscles are the principal
plantarflexors of the foot. Corrective bracing that takes place
especially in the footplate of an orthosis can realign the joints,
elevate the head of the talus and put the calcaneus back into
its appropriate position. In time this will ultimately reduce
the deviated line of pull of the muscle tendons and slowly let
the muscles get back to their normal line of progression. This
is assuming that there is no current permanent damage.

1. The fixed deformity
Fixed deformities are also known as contractures. They are essentially
rigid malalignments of joints. They can be a result of prolonged
malalignment of joints which prevent the normal range of motion
of that joint. Management for fixed deformity is quite difficult
orthotically. Any kind of prevention of further deformity can
be controlled with the proper orthotic system using the proper
corrective force systems. Sometimes over long periods of time,
orthoses can loosen up fixed deformities to a degree.

2. The dynamic deformity
The dynamic deformity consists of a non-fixed malalignment of
the joints. This is the result of an imbalance of the muscle's
tendonous pull. This is the category that most people with CMT
fall under. Most of the time this can be easily corrected with
the proper molding, mold rectification, and an appropriate carbon
graphite device to maintain corrected joint alignment.

Whenever
I receive e-mails or phone calls from CMT'ers, usually someone
says "my balance is getting worse", or "will
these braces help with my balance". Yes, they do help
restore balance. They do this by correcting and stabilizing
the deviations in joint alignment. But how is balance achieved?
It is achieved through forces that stabilize segments of the
body. What is stability in bracing? Stability: the broader
the supporting area the greater the stability becomes.
Greater stability equals

greater balance. Through
careful mechanical design of the orthosis, stability is achieved
by two essential things: 1) the alignment of the orthosis, 2)
the mechanical design of the footplate (the aspect that you bear
weight on).

Designing the orthosis
with stability brings your center of gravity to a more normal
position statically (standing). This directly translates to better
balance while walking. In order to maintain balance in standing
and walking, the body's center of gravity needs to be in the appropriate
location. These laws of physics apply to all things that require
stability in remaining upright (including animals, buildings,
bridges, etc).

Through proper brace
design and reduction of gait deviations, better balance and stability
is attainable for you.

With CMT, we want to
correct your foot as much as possible. What does this mean? This
means within the tolerances that meet acceptable standards. We
do not want to correct your foot beyond what your body can tolerate.
As practitioners, we must realize our limits and your limits as
well. We will work with you to properly design your orthosis,
so that you can wear it comfortably with maximal correction. Fortunately,
silicone allows greater tolerance to correction and greater comfort.

1. To prevent any further
deformity.
2. Correction of any deformity already present.
3. To improve limb function.

At the very least the
primary goal of any orthosis is to prevent any further deformity.
Once this is done new alignment can be put in place and better
balance develops as a result. If you can take care of number one,
and in addition take care of numbers two and three, and put all
three equations together: the patient will experience improved
alignment which is an improved structural outcome. This equals
enhanced balance and usually results in an increased velocity
or walking speed.

After your initial
evaluation at Ortho Rehab Designs your lower limbs will be custom
molded. Then a plaster mold is made of your limbs, and then mold
rectification takes place. This brings us to the diagnostic stage
of your visit. Once the mold is properly rectified with specific
measurements and data from your videotape, a diagnostic orthosis
is then made. The diagnostic orthosis serves as a fitting tool.
From this we get back important information on how to make your
final carbon graphite orthosis. The diagnostic orthosis helps
us determine:
1. Your fit.
2. The proper structural alignment of your limb and joints.
3. Your comfort.

We will work with you
in the diagnostic orthosis for approximately one day. Once we
have determined that the fit, alignment, and comfort are all in
check, we will then go to final fabrication of your carbon graphite
Dynamic Response Orthosis. The diagnostic serves as a prototype
to ensure a proper fitting final device.

When a person's joints
are malaligned, especially when wearing an orthosis, it fatigues
the whole body and causes an increased oxygen consumption. In
effect, what this means is that the person is losing energy while
they ambulate. This is the worst possible thing that could happen
to a brace wearer, as losing energy creates greater fatigue and
in addition can cause further deformity as the body tries to compensate
for this.

For example: when a
foot is derotated, and the knee is taken out of hyperextension,
we get corrected alignment. This corrected alignment linked with
a proper stabilizing fit that uses corrective three-point pressure
systems, will allow the patient to maintain proper alignment and
have a much improved gait. Through proper correction the person
is less fatigued and has greater endurance while walking in their
orthosis, and with the addition of a dynamic release brace, gets
more energy from the entire process.

With the proper corrective
bracing we get increased energy return.

This is what the term
"Creating Corrective Energy" means. I started using
this term when I learned how much energy could be returned to
a brace wearer, once the proper corrections were linked with a
Dynamic Release Orthosis. When a brace user is not in proper alignment
or does not have a proper fit, or proper three point pressure
systems incorporated, that person will lose energy as they walk.
Our goal is to produce energy, energy that corrects and gives
back to the wearer. This energy also increases velocity which
in turn gives better balance and stability.

Thanks for your e-mail. My experience with Mitch was so positive
that I'd gladly respond to your request for more information:

Q. Which Helios brace to you wear?
A. I wear the double Helios style.

Q. Do you wear one or two braces?
A. One on each leg.

Q. The how well do you walk with the braces?
A. The best I have ever walked in my life. No question about it.
I was diagnosed at age 20. Discovered these braces at age 32.
I had so many bad habits created in my gait. These braces, along
with some discipline to remember to straighten my knees when I
walked, changed my gait forever. When I walk now, it is fluid,
and you would not suspect that I have CMT. In fact, to give you
an even more compelling argument, I am now 34 and 7 1/2 months
pregnant. I was very nervous that I would have problems with my
legs and my stability during my pregnancy. With 9 weeks to go,
I still have no problems and my balance is near-perfect. I think
it has to do with a good diet and these Helios braces. Even more
important than my gait improvements, my balance is near-perfect.
I can stand still for hours at a time (when standing on both legs),
not feeling the slightest bit clumsy or off center. (Remember
again—even at 7 months pregnant, my balance hasn’t
changed!).

Q. Has it improved your balance while standing still?
A. Please see above. The results are impressive when you are used
to always having to hold onto something.

Q. Do you need to use a cane?
A. Nope!

Q. Did your medical insurance pay for the braces?
A. Yes. Check out your benefits to see if they cover this type
of durable medical equipment and be persistent. I recommend that
you get everything preapproved, if possible. This doesn't (shouldn't
take very long). Collecting after the fact is very difficult.

Q. How long have you worn the braces?
A. Going on two years in June.

Q. How long did you need to stay in Las Vegas to be fitted?
A. 1 week -- there is so much to do! It was like a mini vacation
for me -- even being alone.

Last but not least, Mitch is very responsive. I live on the East
Coast and he responds very quickly (within 24 hours of my calls
if not sooner). The stellar customer service you receive from
both Mitch and his assistant Michelle make your investment that
much more worthwhile. Any potential problems are addressed promptly.
Michelle also has experience working with insurance companies.
Although I had to file everything myself, she gave me very good
advice throughout the process.

To all those with CMT, who would welcome the ability to walk
with a normal gate:

Over the past eight years, I have tried various types of braces.
I have found this to be a thoroughly exasperating experience.
Physicians lacked knowledge in bracing and orthotists did not
understand the needs of a person with CMT. Also, they lacked the
knowledge of innovative approaches and offered few options (the
‘One Brace Fits All’ concept).

I was searching for a brace that would act as a support for the
muscles that were reduced in their strength and function, and
additionally allow the muscle groups that were still working to
perform.

Also, I wanted a brace that was comfortable, would enable me
to walk with a normal gate, offer stability while standing or
walking, and support proper posture.

If you have ever searched for a brace, you probably find my desires
equivalent to ‘The Impossible Dream'.

My search is over thanks to Mitchell Warner.

Mitchell Warner at Ortho Rehab Designs has developed and trademarked
braces specifically for CMT. He possesses the knowledge and experience
to evaluate and recommend solutions based on an individual’s
needs.

I spent five great days in Las Vegas, NV, receiving the ultimate
in courteous and professional care.

I am now able to out pace a majority of pedestrians in Manhattan.
The Helios Orthosis enables me to stand and walk with stability.
I can navigate uneven surfaces, and climb and transcend slopes.
Achieving all this with absolute comfort and proper body alignment.

Ortho Rehab Designs is a proud supporter of The Hereditary Neuropathy Foundation, an organization dedicated to raising awareness and improving the lives of people with Charcot-Marie-Tooth and other debilitating hereditary neuropathies.